肾脏上皮样血管平滑肌脂肪瘤临床特点  

Clinical characteristics of renal epithelioid angiomyolipoma

作　　者：张大坤 张洪波 董德鑫 高翔 孟强 高纪元 ZHANG Dakun;ZHANG Hongbo;DONG Dexin;GAO Xiang;MENG Qiang;GAO Jiyuan(Department of Urology,Beijing Longfu Hospital,Beijing 100010;Department of Urology,Peking Union Medical College Hospital,CAMS&PUMC,Beijing 100730,China)

机构地区：[1]北京市隆福医院泌尿外科,北京100010 [2]中国医学科学院、北京协和医学院、北京协和医院泌尿外科,北京100730

出　　处：《基础医学与临床》2025年第3期375-377,共3页Basic and Clinical Medicine

基　　金：首都卫生发展科研专项:城市区域中西医结合老年健康服务体系的构建(首发2020-2-7014)。

摘　　要：目的回顾性分析19例肾脏上皮样血管平滑肌脂肪瘤(REAML)诊治病例,探讨其临床特点和诊疗方法。方法通过收集2020年6月至2024年3月收治的19例REAML患者的临床数据,总结其疾病的临床特点、手术方式和随访等临床资料,进行临床特点分析。结果全部19例患者均顺利接受腹腔镜手术治疗,其中16例实施后腹腔镜肾部分切除术,另外3例采取后腹腔镜根治性肾切除术;术后的病理诊断结果均显示为肾脏上皮样血管平滑肌脂肪瘤;经过4~45个月的随访,未发现肿瘤复发以及转移。结论肾脏上皮样血管平滑肌脂肪瘤是临床罕见疾病,具有潜在恶性特征以及复发和转移的风险。通过手术彻底切除肿瘤是治疗的有效手段,优先选择后腹腔镜肾部分切除术。Objective To retrospectively review 19 cases of renal epithelioid angiomyolipoma(REAML)and to explore the clinical characteristics and diagnostic methods in order to optimize diagnosis and clinical treatment.Methods Clinical data of 19 patients with REAML admitted to the hospital from June 2020 to March 2024 were reviewed for clinical characteristics of the disease,surgical procedure and follow-up outcomes.Results All 19 patients received successful laparoscopic surgery.Among them,16 cases received retroperitoneal laparoscopic partial nephrectomy and 3 cases received retroperitoneal laparoscopic radical nephrectomy.Post-operative pathological diagnoses indicated that all cases were classified as renal epithelioid angiomyolipoma.After 4-45 months of follow-up,no tumor recurrence or metastasis was observed.Conclusions Renal epithelioid angiomyolipoma is a rare clinical disease with potential malignant transformation as recurrence and metastasis.Complete resection of the tumor by surgery is an effective treatment.Retroperitoneal laparoscopic partial nephrectomy is the first choice.

关 键 词：肾脏上皮样血管平滑肌脂肪瘤 肾肿瘤 临床特点 

分 类 号：R737[医药卫生—肿瘤]